Sensitivity and specificity of clinical, histologic, and immunologic features in the diagnosis of paraneoplastic pemphigus

Joly, P.; Richard, C A; Gilbert, Danièle; Courville, Philippe; Chosidow, Olivier; Roujeau, Jean‐Claude; Beylot‐Barry, M.; D’Incan, M.; Martel, Ph.; Lauret, P; Tron, François

doi:10.1067/mjd.2000.107488

Cited by 205 publications

(264 citation statements)

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“…Based on Joly and colleagues' study of 22 PNP patients [8], the presence of autoantibodies to periplakin or envoplakin was 82% sensitivity and 100% specificity. However, in a case reported by Sanz-Bueno J, et al, a patient with paraneoplastic autoimmune multiorgan syndrome secondary to a lymphoblastic T-cell lymphoma, had no detectable autoantibodies, who presented with a lichenoid dermatitis and vitiligo, later developing bronchiolitis obliterans and autoimmune hepatitis [31].…”

Section: Treatment Optionsmentioning

confidence: 99%

“…Suprabasal acantholysis and clefts with scattered necrotic keratinocytes are the unique histopathological features. Autoantibody activity in PNP is complex, but usually highly characteristic [2,[6][7][8]. PNP patient sera recognize multiple antigens, which have been identified as the plakin protein family that includes desmoplakin, bullous pemphigoid antigen I, envoplakin and periplakin, and desmogleins 1 and 3.…”

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Paraneoplastic Pemphigus

Zhong-Shuai¹,

Li²

2017

J Dermatol Res Ther

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Paraneoplastic Pemphigus (PNP) is an autoimmune-related acquired bullous disease associated with neoplasia. Both humoral and cellular immunity are involved in the pathogenesis of PNP. Characteristically, PNP has a diverse spectrum of clinical and immunopathological features. Suprabasal acantholysis and clefts with scattered necrotic keratinocytes are the unique histopathological features. PNP patient sera recognize multiple antigens, which have been identified as the plakin protein family. Non-Hodgkin's lymphoma, Castleman's disease, thymoma, follicular dendritic cell sarcoma and chronic lymphocytic leukemia are the commonly associated neoplasms in PNP. The treatment of PNP to date has been rather disappointing. For patients with Castleman's disease, the early detection and removal of the tumor are critical for the treatment of PNP.

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Section: Treatment Optionsmentioning

confidence: 99%

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confidence: 99%

Paraneoplastic Pemphigus

Zhong-Shuai¹,

Li²

2017

J Dermatol Res Ther

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“…Both patterns were seen in our patients. In patients 1 and 3, the results of DIF are negative and thus it is not a necessary diagnostic criterium for PNP [6][7][8]. similar to normal follicular dendritic cells.…”

Section: Direct Immunofluorescencementioning

confidence: 99%

“…Given the strong expression of plakins, rat or monkey urothelium is the most appropriate substrate for screening for antibodies to PNP ( Figure 4) [8,24]. Sera from patients with pemphigus vulgaris or foliaceus do not react with urothelium.…”

Section: Indirect Immunofluorescencementioning

confidence: 99%

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2017

J Dermatol Res Ther

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“…3 However, these autoantibodies have also been found in other diseases, including pemphigus foliaceus (PF), pemphigus vulgaris (PV), bullous pemphigoid (BP), and erythema multiforme major. [4][5][6][7][8][9][10][11][12] A possible mechanism for the development of autoantibodies to DP in those dermatoses is explained by the epitope-spreading phenomenon.…”

Section: Introductionmentioning

confidence: 99%

Analysis of the reactivity of indirect immunofluorescence in patients with pemphigus foliaceus and pemphigus vulgaris using rat bladder epithelium as a substrate

2011

Journal of the American Academy of Dermatology

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OBJECTIVES:To evaluate the reactivity of indirect immunofluorescence using rat bladder epithelium as a substrate in patients with pemphigus foliaceus and pemphigus vulgaris from the Department of Dermatology, University of Sã o Paulo Medical School, Brazil. METHODS:Thirty-two patients (8 male and 24 female) from the Department of Dermatology, University of Sã o Paulo Medical School, were selected. Three had mucosal pemphigus vulgaris, 20 had mucocutaneous pemphigus vulgaris, and 9 had pemphigus foliaceus. Patients' sera were tested by indirect immunofluorescence performed on human foreskin and rat bladder epithelium and by ELISA assays utilizing baculovirus-expressed recombinant desmoglein 3 and desmoglein 1. RESULTS:No patients with mucosal pemphigus vulgaris, 5 of 20 patients with mucocutaneous pemphigus vulgaris (25%) and 4 of 9 patients with pemphigus foliaceus (44%) had positive indirect immunofluorescence using rat bladder epithelium as a substrate.CONCLUSION: Indirect immunofluorescence using rat bladder epithelium as a substrate is recommended whenever a diagnosis of paraneoplastic pemphigus is considered. The identification of a subset of pemphigus foliaceus and pemphigus vulgaris patients that recognizes desmoplakins by this laboratory tool is critical to avoid the misdiagnosis of paraneoplastic pemphigus.KEYWORDS: Pemphigus vulgaris; Paraneoplastic pemphigus; Indirect immunofluorescence; Rat bladder epithelium; Pemphigus foliaceus.Ortolan DG, Souza DPG, Aoki V, Santi CG, Gabbi TVB, Ichimura LMF, et al. Analysis of the reactivity of indirect immunofluorescence in patients with pemphigus foliaceus and pemphigus vulgaris using rat bladder epithelium as a substrate.

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Sensitivity and specificity of clinical, histologic, and immunologic features in the diagnosis of paraneoplastic pemphigus

Cited by 205 publications

References 30 publications

Paraneoplastic Pemphigus

Paraneoplastic Pemphigus

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Analysis of the reactivity of indirect immunofluorescence in patients with pemphigus foliaceus and pemphigus vulgaris using rat bladder epithelium as a substrate

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